Abstract: Background Aicardi Syndrome is an X-linked autosomal recessive neurodegenerative encephalopathy. The diagnostic triad is composed by infantile spasms, agenesis of corpus callosum and chorioretinal lacunae. Additional common findings are: microencephaly, spasticity and severe mental delay. It affects only females because of early embryonic lethality in males. A significant number of females with Aicardi syndrome are of normal birth and develop normally until three months of age when infantile seizures begin. Psychomotor retardation is usually severe, neuromotor retardation is commonly present with lack of motor and language skills. Literature reports only few information about the dentofacial features of the syndrome. Case report We present the case of a patient, by describing the dental and facial characteristics with focus on dental prevention in order to avoid dental pain and the risks connected to general anaesthesia, and ultimately for improving the quality of life.
